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GAA Polyclonal Antibody

Catalog Number:

29700
other_names: GAA; LYAG; glucosidase alpha, acid

Amount:

100μg
calculated_mw: 105kDa
host_species: Rabbit

Price:

$319

Swiss-Prot No:

Swiss-Prot#:P10253
NCBI Gene ID:2548

Form of Antibody:

Avoid freeze / thaw cycles.|Buffer: PBS with 50% glycerol, pH7.4.

Storage/Stability:

Immunogen:

A synthetic peptide of human GAA (NP_000143.2).

Purification:

Affinity purification

Specificity/Sensitivity:

Applications:

WB,IF

Background:

This gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme. Defects in this gene are the cause of glycogen storage disease II, also known as Pompe's disease, which is an autosomal recessive disorder with a broad clinical spectrum. Alternative splicing results in multiple transcript variants.

References:

appl_detail:

WB 1:500 - 1:2000
IF 1:50 - 1:200
IP 1:50 - 1:200

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